Strange Week 28th June 2011

Strange Week 28^th June 2011

As the title suggests it has been a bit of a strange week, where Robbie seems to be struggling a bit more than usual. He seems to be suffering with pain in his shoulders, back and legs and has also been very moody.

Currently for pain relief we are a bit limited (usually Calpol and hot baths) these seem to soothe the symptoms albeit very slightly.

Sometimes you ask yourself, is it just because we are getting nearer the time for the next visit to Evelina that his symptoms seem worse, or is it, as both my Wife and I believe, genuinely getting worse.

Parents with children with Dystonia will associate with the feeling of helplessness that is felt when their children complain of the pain they are feeling, and the lack of assistance you can give your child, luckily for us Robbie is only six, and a close cuddle from Mum or Dad still help to soothe him immensely.

Went to the school today with the literature we received from the Evelina on our visit at the start of last week, the idea was that Robbie’s classroom assistant, teacher and head teacher could have a read over and jot down any questions they may have in relation to Robbie’s school requirements post op…. as these are raised we will speak to the team at the Evelina and gather answers to any queries they may have. In a similar fashion we are composing an ever growing list of questions to ask the team when we go down late July which we would like answers on… Probably the one at the front of my mind just now is will he be able to stop taking all these tablets every day?

The clock seems to be slowing down the closer we get to these appointments, I take it this may well be a mixture of just wanting this to happen sooner rather than later, along with that bit of fear of the unknown on how successful the Op will be.

At this stage I need to say a huge “Thank You” to the kind parents of other children who have had this surgery and taken the time to show their support and encouragement. There really are some amazing success stories out there, trust me these words of experience are making this journey so much easier to deal with… I thank all of you… You know who you are.

Luckily for us we have a little holiday between the 8^th – 15^th July, before we head back down,  when hopefully we will be distracted from what lies ahead of us… This is going to be the best holiday ever… As Robbie says “Only 10 sleeps till we go” J

Medications 23rd June 2011

Since a bit of interest in this blog has been shown by other parents and families who have been in, or believe they are starting to experience a similar situation I think it would be good to touch on a question that has arisen from many parties.

Medications - What medication is Robbie on ?

• Sinemet (Co-Careldopa 12.5MG/50MG) –

 Robbie was given a drug trial with Sinemet about October 2010 (Just after his diagnosis) initially he was prescribed  one a day for the first week, this increased to two a day the second week, and after a couple of further weeks this increased to three a day (Morning, Lunchtime and Teatime) There was a slight improvement in Robbie’s mobility during this time, albeit he still struggled day to day. Robbie still takes three doses a day, not sure if this is something that will need to continue once his DBS is done (another question to be asked on our next visit to the Evelina team !)

• Trihexyphenydil – 2MG

Robbie was prescribed these a month or two after the Sinemet was originally prescribed, and the tablets were taken in conjunction with Sinemet. Again, initially he was prescribed one half a tablet twice daily, which increased to three quarters of a tablet twice a day (Breaking a tablet into quarters isn’t such an easy task by the way!) There was (In Robbie’s case anyway) again slight further improvement, and the dosage was increased to one tablet twice daily, this was further increased again to two tablets twice daily  however we did experience an issue a few weeks in where Robbie kept feeling like he needed to urinate, however when he tried he said (In his words – “It’s not coming”) we discussed this with his neurologist who advised this was a possible side effect of the medication and the dosage was reduced to one and a half a day, and this seemed to resolve this issue very quickly, over time we noticed Robbie kept rubbing his eyes and nose on a very regular basis, and he said his eyes were funny, again the neurologist was advised of this and again it turned out blurred vision was another side effect of this medication, and Robbie’s dosage was cut to one tablet twice daily.

Robbie is unbelievably good for a 6 yearold at taking his medication, and often reminds us it is time for his tablet...

So in summary Robbie is currently taking the following medication on a daily basis –

Sinemet (1 tablet x 3 times daily)

Trihexyphenydil (1.5 tablets x 2 times daily)

Ongoing side effects - One comment I would add is Robbie can become very moody and deep since the introduction of his medication, whereas he was always a very happy go lucky lad prior to taking his meds, there can now be occasions where he is his old self, but there are also other times when he becomes very easily upset, has dramatic tantrums and general mood swings, that said this could just be him becoming increasingly aware of things he cannot do as easily as other children his age, then again it could just be our little boy getting older and developing his own personality...

 Is the medication causing this ? Only time will tell...

So Tired 21st June 2011

Robbie was back at school today, fresh from the trip he was up early and raring to go.. dropped off at his usual time he was happy to be back in familiar surroundings again.

School were updated on the previous couple of days and a meeting has been requested with the school as part of his integrated support plan... we figured it best to have a sit down with the school and go over the literature from the Evelina and give them a chance to look at this and establish if the school had any questions they needed answered in relation to Robbie's procedure.

The thinking is along the lines of what post op assistance Robbie may need when he is back at school after the summer holidays... My thinking is this will probably be a wait and establish the success of the op first, but it is best to keep these people in the loop.

Robbie was very tired when he got in from the school, and was feeling sore (back, arms and legs) he basically came in and had a nice warm bath, and pretty much lay on the couch till about 6.30pm when he announced he wanted to go to bed !!!

Robbie fell asleep very quickly and we can only guess the travel over the last few days had worn him out.

For those who may be unaware when your child has Dystonia the school does have a support plan (this applies to any condition a child may suffer from being it physical or mental) and I would wholeheartedly recommend any parent pushes for this if required. Basically an integrated support plan brings together both educational and medical professionals involved in any treatment or needs your child may have and ensures these are catered for whilst the child is at school.

Evelina - Initial Visit - 20th June 2011

So here we are, our first big adventure trip to London and the big city, off down to the Evelina and meet the team...

We really were pretty uncertain on what to expect, and felt very nervous at 9am in the morning for our 9.30am appointment we entered what seemed a huge modern building, full of strange unfamiliar but friendly faces.
The staff in general seemed really happy and comfortable in their workplace, everybody seemed to smile and say hello, this was very re-assuring for both Robbie and I.

We were called into a consultation room and we were introduced to Dr Lin (Consultant Paediatric Neurologist) Sarah Perides (Paediatric Nurse Co-Ordinator) Kylee Tustin (Clinical Specialist Paediatric Physiotherapist) and Lesley Baker (Senior Paediatric Speech And Language Therapist)

Initially Robbie was very quiet and seemed a bit phased by the people who sat with us, however all were very friendly and spoke to Robbie, and he soon relaxed and took part in the conversation.

Before we attended the Evelina for our first visit we had completed a questionnaire about Robbie and it was very apparent the team had looked very closely at the comments we had wrote, as several of their questions were based around the answers we had gave.

The team also got us to talk about the history of the condition, what had happened, what were his first symptoms, how had they progressed over time... all the way through this process notes were taken, and unusually they seemed to be taking on board every bit of information they were being given.

After an hour or so, I eventually stopped talking (LOL) and they raised any questions they had into what I had been saying.

Next Dr Lin went on to explain about deep brain stimulation and how the implant can help, this was done in an easy to understand manner, which helped immensely.

Next Robbie was put through a series of tests including walking, running, stretching, grasping exercises to name but a few... he enjoyed these, and the team made them a game, which Robbie enjoyed.

Next the team went in to quite a bit of detail about deep brain stimulation and what it entailed, giving a very clear roadmap on to the next step of the journey, which is a 5 day series of tests and assessments including goal setting for the DBS.

They explained how DBS can help young children with Dystonia in many ways - such as those set out below...

• Relieve Dystonia and associated pain
• Improve Movement Control
• Improve sitting posture and tolerance
• Improve sleeping pattern
• Increase Personal Independance, participation and self confidence

This is an exhaustive list, however above are the main areas I believe will benefit Robbie

There are a variety of tests planned for our 5 day visit at the end of July, and all areas were covered during this meeting, they discussed goal setting and have asked us to have a think about Robbie and what areas we would most like to see an improvement in, they covered when the DBS would take place, where the DBS would take place, who will perform the surgery (along with stats on success and improvement rates), what the surgery involved, what the stimulator would look like... (amazingly they actually had a stimulator and battery pack I could touch and hold, and they showed us x-ray pictures of implanted stimulators, and discussed how the surgery would be performed and aftercare Robbie would receive. This was a truly enlightening experience which helped alleviate  a lot of fears, however also gave us some real food for thought...

Truly your head will be spinning from the amount of information that is given, however on the positive side they gave us an information pack which is very conclusive and covers a variety of questions you may well have. This is a great source of information and actually allows you to look at what was discussed and really gives you a chance to absorb some of the information you have been given on the day, as I/We have read over this new questions have come to mind, now though I am writing these down as and when they spring into my mind (thus this document will no doubt be read several times over before our next visit)

They also have a book for the child entitled "Tafi's DBS Story" this is a very child friendly book, very bright and cheery colours, however also covers the procedure in full, this will be really beneficial to Robbie in the lead up to both the 5 day visit and the procedure itself.

Overall trust me you have absolutely nothing to fear if you are going to meet this team, they are a truly fantastic group of  medical staff, who certainly made us feel at ease very quickly, they certainly showed a real understanding of the condition - and most of all we knew we were in very safe hands, with the child's welfare being foremost in all of their minds.

PS... Robbie was happy with the information given... apart from the part where they said he needed to shave his hair for the surgery (Guess he will have hair like Dad's for a short while :)

Gassiot House 19th June 2011

We spent the night in Gassiot House after taking a taxi from Kings Cross, I am afraid the thought of using the tube wasn't at all appealing with Robbie, his chair and our luggage all taking their toll...
The taxi driver dropped us off at the doors of the Evelina, however I was still to discover Gassiot House isn't exactly next to the Evelina... In fairness it isn't really far (maybe it just seems so when you haven't got the foggiest idea where you are.)

We made our way through St Thomas hospital as directed by the  very friendly security chap, and once we had navigated our way to the front entrance of the hospital asked a further security guard for further directions, sure enough another very friendly face who pointed me n te direction of Gassiot House...

Here lies a warning, if you arrive out of normal office hours, you will need to go speak to the security chap at the St Thomas front desk to pick up your keys !!!....

We found our way in and a sense of calm descended... at least we knew we were there, and had safe, warm accommodation for the night. The accomodation is split into flats (which contain three double bed rooms, a shared kitchen, lounge and two bathroom suites)....(best to note they even provided towels, to save you having to haul these with you)

Gassiot House was overall immaculate, very quiet (considering location) and more than enough for any short visit....

Childhood Onset Dystonia 18th June 2011

This story is purely based on my 6 year old son Robbie, Robbie suffers from Idiopathic Generalised Childhood Onset Dystonia and as we are now leading up to a possible deep brain implant, I will regularly be stopping by to update his story and progress...

I think we need to start out with what has happened with Robbie to date and then this blog will be updated as we no doubt wander down this yet to be known course of treatment and the doubtless rollercoaster of emotions we as Robbie's parents go through up to the outcome (who knows how long this process will take)

My aim is to give any parents in the future, who may go through a similar experience a help to understand they are not alone on this frankly horrific and mind numbing condition. It has been the most difficult year of our lives watching what was a healthy and normally happy 4 year old spiral downhill, and have problems with motor co-ordination, however we have been given fresh hope for Robbie with this relatively new experimental surgery

It is 19th June 2011 and here is where we are so far...

Robbie was 3years 10 months when we first took him to our GP as his hand seemed to be constantly drawing in to his body, he is now 6 years old, the doctor referred us to a GP at our local hospital, in time the specialist at our local hospital kept sending Robbie for epilepsy tests every few months and it became apparent over time the consultant did seem stumped and could find no diagnosis or reason for this happening, we were advised one and a half years later by the consultant throughout which time Robbie was subjected to a variety of tests, the consultant  advised that he believed the condition was non-progressive, and as such Robbie was assigned to an occupational therapist to try and develop his fine motor skills.

A couple of months later Robbie developed an issue with his right leg and we arranged another visit to the consultant, who again tried to send Robbie for EEG tests, and began looking at his bloods and eyes for answers, as we painfully watched Robbie worsen rapidly with both legs soon being affected I wrote to the complaints department at my health board as we had now been hanging on for two years, obviously this was a most stressful time for us as parents as we had no indication of either a diagnosis or prognosis of his condition. Robbie now has a wheelchair which we use if going any distances etc as he struggles to walk very far.

After we raised the complaint with our local health board Robbie was rapidly passed over to a neurologist at the Sick Kids in Edinburgh, Dr Eunson who put Robbie through several tests and investigations, and eventually after ruling out many potentially worrying conditions we were advised Robbie has idiopathic early childhood onset dystonia. This has now further led to Robbie being administered medicines, with a view to possible deep brain stimulation with Dr Linn in London.... which is where we currently stand...

Robbie has since been referred to the Evelina Hospital Childrens Hospital in London... and this is where the blog begins... This will be updated on a regular basis and will serve as both a diary to myself and any others who may want to occasionally pop by to read...

(6pm) - 10.06.11 - An article which had been picked up by STV (Scottish televison) news was broadcast across Scotland, in this the aim was to raise awareness of this mainly unrecognised condition, watch if you like here... http://news.stv.tv/scotland/east-central/256563-boys-family-campaign-for-better-recognition-of-dystonia/

(6am) - 19.06.11 - Up at 5am, stomach churning thinking of the trip from Kirkcaldy in Fife, Scotland all the way down to London.... Robbie and the rest of the family still lying sleeping. So a bit of quiet time to sit and reflect on what lies ahead of us, Deep Brain Stimulation sounds a good option, to be fair after several months on medication it is pretty much our last option, so (and I am not a religious type of guy by any manner) I pray this will get results we need for Robbie to have a better quality of life...
This will be the first night we have never spent together for all my family, sounds strange in this day and age, but we are a very tight, close family unit... and to be honest, deep down I know it will be difficult.